School of Public Health, Xi'an Jiaotong University Health Science Center, Key Laboratory of Trace Elements and Endemic Diseases, National Health and Family Planning Commission, No. 76 Yanta West Road, Xi'an, 710061, Shaanxi, People's Republic of China.
Xijing Hospital, Medical University of the Air Force, Xi'an, 710061, Shaanxi, People's Republic of China.
Glycoconj J. 2019 Dec;36(6):451-459. doi: 10.1007/s10719-019-09889-9. Epub 2019 Sep 2.
Kashin-Beck disease (KBD) is an endemic degenerative osteoarthropathy of uncertain etiology. Our study sought to identify a correlation between small proteoglycans decorin and biglycan expression and Kashin-Beck Disease. Immunohistochemistry was used to assess the decorin and biglycan levels in cartilage specimens from both child KBD patients, and rats fed with T-2 toxin under a selenium-deficient condition. Real-time PCR and Western blot were used to assess mRNA and protein levels of decorin and biglycan in rat cartilages, as well as in C28/I2 chondrocytes stimulated by T-2 toxin and selenium in vitro. The result showed that decorin was reduced in all zones of KBD articular cartilage, while the expression of biglycan was prominently increased in KBD cartilage samples. Increased expression of biglycan and reduced expression of decorin were observed at mRNA and protein levels in the cartilage of rats fed with T-2 toxin and selenium- deficiency plus T-2 toxin diet, when compared with the normal diet group. Moreover, In vitro stimulation of C28/I2 cells with T-2 toxin resulted in an upregulation of biglycan and downregulation of decorin, T-2 toxin induction of biglycan and decorin levels were partly rescued by selenium supplement. This study highlights the focal nature of the degenerative changes that occur in KBD cartilage and may suggest that the altered expression pattern of decorin and biglycan have an important role in the onset and pathogenesis of KBD.
大骨节病(KBD)是一种病因不明的地方性退行性骨关节病。本研究旨在探讨核心蛋白聚糖(decorin)和 biglycan 表达与大骨节病之间的相关性。采用免疫组织化学法检测儿童大骨节病患者和硒缺乏条件下 T-2 毒素喂养大鼠软骨标本中 decorin 和 biglycan 的水平。采用实时 PCR 和 Western blot 检测大鼠软骨和 T-2 毒素及硒体外刺激的 C28/I2 软骨细胞中 decorin 和 biglycan 的 mRNA 和蛋白水平。结果表明,KBD 关节软骨各层的 decorin 均减少,而 biglycan 在 KBD 软骨标本中的表达明显增加。与正常饮食组相比,T-2 毒素和硒缺乏加 T-2 毒素饮食喂养的大鼠软骨中 biglycan 的表达增加,decorin 的表达减少,在 mRNA 和蛋白水平上均观察到这一结果。此外,T-2 毒素体外刺激 C28/I2 细胞导致 biglycan 上调,decorin 下调,硒补充部分挽救了 T-2 毒素诱导的 biglycan 和 decorin 水平的升高。本研究强调了 KBD 软骨退行性变化的局灶性特征,可能表明 decorin 和 biglycan 的表达模式改变在 KBD 的发病机制中起重要作用。